Case and Research Letters
Systemic Follicular Lymphoma With Cutaneous Manifestations and Exclusively Cutaneous Recurrence
Linfoma folicular sistémico con afectación cutánea y recidiva únicamente cutánea
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Palacios Abufón, E. Acebo Mariñas, J. Gardeazabal García, J.C. García-Ruiz" "autores" => array:4 [ 0 => array:4 [ "nombre" => "A." "apellidos" => "Palacios Abufón" "email" => array:1 [ 0 => "andres.palacios.ib@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "E." "apellidos" => "Acebo Mariñas" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "J." "apellidos" => "Gardeazabal García" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "J.C." "apellidos" => "García-Ruiz" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario de Cruces, Baracaldo, Vizcaya, Spain" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Hematología, Hospital Universitario de Cruces, Baracaldo, Vizcaya, Spain" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Linfoma folicular sistémico con afectación cutánea y recidiva únicamente cutánea" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1338 "Ancho" => 993 "Tamanyo" => 389853 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Bcl2<span class="elsevierStyleSup">+</span> lymphoid infiltrate.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Follicular lymphoma (FL) constitutes approximately 30% of all non-Hodgkin lymphomas in Western countries. Clinical presentation is typically in the form of lymphadenopathy, hepatomegaly, splenomegaly and bone marrow infiltration.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Extranodal involvement is less frequent than in large B-cell lymphoma<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and does not appear to affect prognosis; this is not the case with B-cell lymphoma.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a patient diagnosed with FL who developed skin nodules in the course of her disease. The lymphoma recurred after treatment, but it was exclusively limited to the skin, an unusual observation in this disease.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was a 54-year-old woman who was referred to the hematology department in August 2004 for thrombocytopenia detected during a routine blood analysis (platelet count 70×10<span class="elsevierStyleSup">9</span>/L). Monoclonal B-cell lymphocytosis in the peripheral blood and bone marrow, enlarged subcentimeter abdominal lymph nodes and splenomegaly were observed during the study. The diagnosis was chronic B-cell lymphoproliferative syndrome and the patient was offered splenectomy, which she refused at that time. At follow-up in January 2007, enlarged mediastinal and retroperitoneal lymph nodes (≤5<span class="elsevierStyleHsp" style=""></span>cm in diameter) were observed, prompting the performance of diagnostic and therapeutic splenectomy. Histologic study of the spleen showed a proliferation of small lymphoid cells, with a micronodular growth pattern, predominantly in the germinal centers of the white pulp, with infiltration of the red pulp. The cells had a CD20<span class="elsevierStyleSup">+</span>, Bcl2<span class="elsevierStyleSup">+</span>, Bcl6<span class="elsevierStyleSup">+</span>, IgD<span class="elsevierStyleSup">−</span> CD23<span class="elsevierStyleSup">−</span>, cyclin D1<span class="elsevierStyleSup">−</span> and p53<span class="elsevierStyleSup">−</span> phenotype and a low proliferative index, findings which were consistent with the diagnosis of FL.</p><p id="par0020" class="elsevierStylePara elsevierViewall">In March 2007, the patient was referred to the dermatology department due to the gradual appearance of asymptomatic subcutaneous nodular lesions on the face, chest and back. The nodules had an erythematous-violacious appearance, were slightly raised, and had diameters ranging between 8<span class="elsevierStyleHsp" style=""></span>mm and 20<span class="elsevierStyleHsp" style=""></span>mm (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Biopsy of the lesions showed a lymphoid infiltrate composed of small monomorphous cells, compatible with FL infiltration (CD20<span class="elsevierStyleSup">+</span>, Bcl2<span class="elsevierStyleSup">+</span>, Bcl6<span class="elsevierStyleSup">+</span>) (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Reassessment of the disease at this time also demonstrated the development of lymphadenopathy, for which chemotherapy was started with a rituximab-CHOP schedule (cyclophosphamide, vincristine, adriamycin and prednisone). Complete remission was achieved after 6 cycles, with clearance of the subcutaneous nodules and the enlarged lymph nodes; subsequently, the patient was placed on maintenance therapy with rituximab administered every 3 months. In December 2009, the patient again presented with cutaneous lesions, predominantly facial, similar to those previously described; biopsy reconfirmed the diagnosis of infiltration by FL. The rituximab maintenance treatment ended in June 2010; the skin lesions were still stable and there was no evidence of disease spread to other areas (peripheral blood immunophenotyping and cervical-thoracic-abdomen CT scan were normal).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">FL is characterized by an indolent course and has no standardized treatment; a variety of approaches are used, including simple observation, radiotherapy, immunotherapy, polychemotherapy, maintenance treatment with rituximab,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> and even the application of various hematopoietic stem cell transplantation treatment modalities.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">As in other lymphoid malignancies, patients with FL can have extranodal involvement affecting the skin in up to 3.8% of cases.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Extranodal involvement in FL does not generally affect overall survival, unlike the case with large diffuse B-cell lymphoma; these 2 diseases constitute the majority of B-cell lymphomas in Spain.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> It is therefore important to rule out transformation of FL to more aggressive forms as these are associated with significantly decreased survival.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> For this reason, lesions should be biopsied whenever a patient experiences relapse or disease progression.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Differential diagnosis is particularly important in primary cutaneous follicular center lymphoma, since it shares histologic features with FL. In addition to systemic involvement, Bcl2 negativity, which is common in cases primarily involving the skin, may be of use in differentiating the 2 diseases.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">FL usually responds well to treatment, although relapses are common and usually affect the lymph nodes, bone marrow or peripheral blood.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> What is unusual is recurrence being limited to the skin, as in our patient. At present, and after more than 6 years of follow-up, the patient's disease remains limited to the skin. The persistence of cutaneous disease reflects a lack of complete control of the disease, and it is therefore likely that the patient will develop systemic disease in the future.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Palacios Abufón A, et al. Linfoma folicular sistémico con afectación cutánea y recidiva únicamente cutánea. Actas Dermosifiliogr. 2012;103:253–55.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 830 "Ancho" => 993 "Tamanyo" => 112437 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Nodule on the chest.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1338 "Ancho" => 993 "Tamanyo" => 389853 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Bcl2<span class="elsevierStyleSup">+</span> lymphoid infiltrate.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Follicular lymphomas" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "U. 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Salles" 1 => "J.F. Seymour" 2 => "F. Offner" 3 => "A. Lopez-Guillermo" 4 => "D. Belada" 5 => "L. Xerri" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/S0140-6736(10)62175-7" "Revista" => array:6 [ "tituloSerie" => "Lancet" "fecha" => "2011" "volumen" => "377" "paginaInicial" => "42" "paginaFinal" => "51" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21176949" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinicopathologic spectrum of cutaneous manifestations in systemic follicular lymphoma. A study of 11 patients" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "K. Dabski" 1 => "P. Banks" 2 => "R.K. Winkelmann" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Cancer" "fecha" => "1989" "volumen" => "64" "paginaInicial" => "1480" "paginaFinal" => "1485" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/2789095" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Reclassification of 300 primary cutaneous B-cell lymphomas according to the new WHO-EORTC classification for cutaneous lymphomas: comparison with previous classifications and identification of prognostic markers" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "N.J. Senff" 1 => "J.J. Hoefnagel" 2 => "P.M. Jansen" 3 => "M.H. Vermeer" 4 => "J. van Baarlen" 5 => "W.A. Blokx" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1200/JCO.2006.09.6396" "Revista" => array:6 [ "tituloSerie" => "J Clin Oncol" "fecha" => "2007" "volumen" => "25" "paginaInicial" => "1581" "paginaFinal" => "1587" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17353548" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000010300000003/v1_201304241257/S1578219012001059/v1_201304241257/en/main.assets" "Apartado" => array:4 [ "identificador" => "6169" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Cases and Research Letters" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000010300000003/v1_201304241257/S1578219012001059/v1_201304241257/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219012001059?idApp=UINPBA000044" ]
| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 20 | 11 | 31 |
| 2024 October | 177 | 65 | 242 |
| 2024 September | 152 | 25 | 177 |
| 2024 August | 147 | 58 | 205 |
| 2024 July | 131 | 48 | 179 |
| 2024 June | 115 | 96 | 211 |
| 2024 May | 128 | 46 | 174 |
| 2024 April | 114 | 29 | 143 |
| 2024 March | 179 | 29 | 208 |
| 2024 February | 181 | 38 | 219 |
| 2024 January | 462 | 38 | 500 |
| 2023 December | 394 | 17 | 411 |
| 2023 November | 545 | 24 | 569 |
| 2023 October | 679 | 39 | 718 |
| 2023 September | 617 | 42 | 659 |
| 2023 August | 673 | 17 | 690 |
| 2023 July | 645 | 35 | 680 |
| 2023 June | 502 | 27 | 529 |
| 2023 May | 312 | 32 | 344 |
| 2023 April | 154 | 19 | 173 |
| 2023 March | 134 | 39 | 173 |
| 2023 February | 278 | 22 | 300 |
| 2023 January | 501 | 34 | 535 |
| 2022 December | 260 | 49 | 309 |
| 2022 November | 214 | 28 | 242 |
| 2022 October | 346 | 30 | 376 |
| 2022 September | 402 | 61 | 463 |
| 2022 August | 238 | 53 | 291 |
| 2022 July | 128 | 37 | 165 |
| 2022 June | 58 | 35 | 93 |
| 2022 May | 154 | 42 | 196 |
| 2022 April | 254 | 37 | 291 |
| 2022 March | 321 | 61 | 382 |
| 2022 February | 234 | 36 | 270 |
| 2022 January | 221 | 45 | 266 |
| 2021 December | 122 | 39 | 161 |
| 2021 November | 183 | 48 | 231 |
| 2021 October | 142 | 50 | 192 |
| 2021 September | 111 | 35 | 146 |
| 2021 August | 104 | 45 | 149 |
| 2021 July | 107 | 29 | 136 |
| 2021 June | 106 | 28 | 134 |
| 2021 May | 76 | 38 | 114 |
| 2021 April | 181 | 70 | 251 |
| 2021 March | 103 | 27 | 130 |
| 2021 February | 96 | 26 | 122 |
| 2021 January | 53 | 19 | 72 |
| 2020 December | 62 | 16 | 78 |
| 2020 November | 48 | 16 | 64 |
| 2020 October | 44 | 12 | 56 |
| 2020 September | 32 | 10 | 42 |
| 2020 August | 48 | 23 | 71 |
| 2020 July | 23 | 15 | 38 |
| 2020 June | 37 | 32 | 69 |
| 2020 May | 23 | 17 | 40 |
| 2020 April | 27 | 21 | 48 |
| 2020 March | 25 | 15 | 40 |
| 2020 February | 6 | 1 | 7 |
| 2020 January | 4 | 0 | 4 |
| 2019 December | 8 | 4 | 12 |
| 2019 November | 4 | 0 | 4 |
| 2019 October | 0 | 20 | 20 |
| 2019 September | 8 | 0 | 8 |
| 2019 August | 4 | 1 | 5 |
| 2019 July | 2 | 3 | 5 |
| 2019 June | 6 | 2 | 8 |
| 2019 May | 3 | 18 | 21 |
| 2019 March | 2 | 7 | 9 |
| 2019 January | 2 | 0 | 2 |
| 2018 December | 3 | 0 | 3 |
| 2018 November | 2 | 0 | 2 |
| 2018 October | 2 | 0 | 2 |
| 2018 September | 3 | 0 | 3 |
| 2018 March | 5 | 0 | 5 |
| 2018 February | 82 | 3 | 85 |
| 2018 January | 84 | 4 | 88 |
| 2017 December | 70 | 7 | 77 |
| 2017 November | 47 | 3 | 50 |
| 2017 October | 63 | 5 | 68 |
| 2017 September | 68 | 2 | 70 |
| 2017 August | 83 | 8 | 91 |
| 2017 July | 58 | 4 | 62 |
| 2017 June | 78 | 7 | 85 |
| 2017 May | 67 | 5 | 72 |
| 2017 April | 66 | 5 | 71 |
| 2017 March | 51 | 11 | 62 |
| 2017 February | 44 | 11 | 55 |
| 2017 January | 43 | 8 | 51 |
| 2016 December | 49 | 7 | 56 |
| 2016 November | 110 | 9 | 119 |
| 2016 October | 62 | 7 | 69 |
| 2016 September | 72 | 19 | 91 |
| 2016 August | 60 | 11 | 71 |
| 2016 July | 36 | 9 | 45 |
| 2016 June | 9 | 15 | 24 |
| 2016 May | 6 | 13 | 19 |
| 2016 April | 9 | 12 | 21 |
| 2016 March | 10 | 2 | 12 |
| 2016 February | 12 | 2 | 14 |
| 2016 January | 9 | 0 | 9 |
| 2015 December | 9 | 2 | 11 |
| 2015 November | 42 | 2 | 44 |
| 2015 October | 40 | 0 | 40 |
| 2015 September | 12 | 6 | 18 |
| 2015 August | 10 | 5 | 15 |
| 2015 July | 97 | 9 | 106 |
| 2015 June | 74 | 6 | 80 |
| 2015 May | 100 | 10 | 110 |
| 2015 April | 81 | 9 | 90 |
| 2015 March | 84 | 6 | 90 |
| 2015 February | 114 | 17 | 131 |
| 2015 January | 87 | 20 | 107 |
| 2014 December | 86 | 7 | 93 |
| 2014 November | 58 | 13 | 71 |
| 2014 October | 79 | 10 | 89 |
| 2014 September | 69 | 9 | 78 |
| 2014 August | 73 | 12 | 85 |
| 2014 July | 77 | 23 | 100 |
| 2014 June | 103 | 14 | 117 |
| 2014 May | 100 | 17 | 117 |
| 2014 April | 62 | 14 | 76 |
| 2014 March | 33 | 7 | 40 |
| 2014 February | 30 | 5 | 35 |
| 2014 January | 25 | 10 | 35 |
| 2013 December | 13 | 10 | 23 |
| 2013 November | 17 | 10 | 27 |
| 2013 October | 16 | 6 | 22 |
| 2013 September | 9 | 5 | 14 |
| 2013 August | 10 | 11 | 21 |
| 2013 July | 11 | 19 | 30 |
| 2013 June | 13 | 20 | 33 |
| 2013 May | 17 | 11 | 28 |
| 2013 April | 15 | 8 | 23 |
| 2013 March | 21 | 12 | 33 |
| 2013 February | 40 | 7 | 47 |
| 2013 January | 88 | 8 | 96 |
| 2012 December | 32 | 5 | 37 |
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